Figure 4. Revised DSRCT Treatment algorithm adapted by MDACC.

The diagnosis of DSRCT is confirmed using immunohistochemistry and/or cytology, and the staging workup is completed. All patients receive neoadjuvant chemotherapy, usually six cycles of the current standard-of-care for ES. Clinical response is assessed with PET/CT every two cycles and the small minority that fail to respond to chemotherapy are transitioned to second-line regimens. Patients that harbor extra-peritoneal disease (EPD) after 6 neoadjuvant chemotherapy cycles are considered poor surgical candidates and continue with chemotherapy. Those lacking EPD proceed to complete cytoreductive surgery (CCS), with the aim of removing all measurable disease. In light the current study findings, we recommend HIPEC only in conjunction with an ongoing clinical study. Whole abdominopelvic intensity-modulated radiotherapy (WART), if provided, is also given as part of a prospective clinical trial designed to assess disease-free survival and intra-abdominal recurrence-free survival. Adjuvant chemotherapy with temozolomide/irinotecan or other agents are provided and oligo-metastatic sites are treated, when needed, using radiofrequency ablation or stereotactic radiation. VAC/IE = Vincristine, Adriamycin, Cyclophosphamide, Ifosfamide and Etoposide; VAI = Vincristine, actinomycin & ifosfamide; VIDE = Vincristine, Adriamycin, Ifosfamide and Etoposide; P6 = 7 cycles of IE/VDC (Ifosfamide, Etoposide, Vincristine, Adriamycin, and Cytoxan); LN = lymph nodes; HIPEC = hyperthermic peritoneal perfusion of chemotherapy; IGF-1R = insulin-like growth factor receptor 1; mTOR = mammalian target of Rapamycin.